Coussa Razek Georges, Kim James, Traboulsi Elias I
Faculty of Medicine, McGill University, Montreal, Quebec, Canada.
Ophthalmic Genet. 2012 Jun;33(2):66-73. doi: 10.3109/13816810.2011.623261. Epub 2011 Nov 7.
BACKGROUND/AIMS: The extent and time course of vision loss in Choroideremia (CHM) is still unclear. We undertook this study to quantitate the change in visual acuity (VA) over time in order to gain a better understanding of the natural course of this retinal disorder.
Corrected VA of 120 males with CHM and 53 female carriers were collected from 24 studies and/or case reports published between 1981 and 2010, as well as from data on 15 patients examined at the Cole Eye Institute (Cleveland Clinic). Cross-sectional and longitudinal analyses were used to investigate the relationship between VA and age, as well as the progression rate of VA with age, respectively. Age grouping effects were investigated using ANOVA.
The mean age of affected males was 36.6 ± 17.7 years. The mean logMAR VA was 0.35 ± 0.53. There was a significant 0.0072 decrease in logMAR VA per year (p = 1.22 × 10(-4)). There was a significant difference between VA of patients <50 years of age and those >50 years (0.27 ± 0.39 vs. 0.61 ± 0.81, p = 2.90 × 10(-5)). When we compared the rate of VA loss for patients <50 years vs. those >50 years, we also found a significant difference (0.01 ± 0.04 vs. 0.06 ± 0.08, p = 1.23 × 10(-2)). The average age of female carriers was 36.4 ± 17.7 years, with an average logMAR VA of 0.36 ± 0.6. There was no significant correlation between VA of female carriers and age (p = 0.12) with 46% of female carriers having a VA better than 20/20 at an average age of 33 years compared to 25% of affected males at 30 years.
In affected males with CHM, VA decreases very slowly until subjects reach 50 years of age, at which time the rate and extent of vision loss become significantly higher. Additionally, VA decreases more rapidly as individuals get older. In contradistinction to affected males, VA loss in female carriers is much milder.
背景/目的：脉络膜视网膜病变（CHM）导致视力丧失的程度和时间进程仍不明确。我们开展这项研究以量化视力（VA）随时间的变化，以便更好地了解这种视网膜疾病的自然病程。
从1981年至2010年间发表的24项研究和/或病例报告中，以及从科尔眼科研究所（克利夫兰诊所）检查的15例患者的数据中，收集了120例患有CHM的男性和53例女性携带者的矫正视力。分别采用横断面分析和纵向分析来研究视力与年龄之间的关系，以及视力随年龄的进展率。使用方差分析研究年龄分组效应。
受影响男性的平均年龄为36.6±17.7岁。平均logMAR视力为0.35±0.53。logMAR视力每年显著下降0.0072（p = 1.22×10⁻⁴）。年龄小于50岁的患者与年龄大于50岁的患者的视力存在显著差异（0.27±0.39对0.61±0.81，p = 2.90×10⁻⁵）。当我们比较年龄小于50岁与年龄大于50岁的患者的视力丧失率时，也发现了显著差异（0.01±0.04对0.06±0.08，p = 1.23×10⁻²）。女性携带者的平均年龄为36.4±17.7岁，平均logMAR视力为0.36±0.6。女性携带者的视力与年龄之间无显著相关性（p = 0.12），46%的女性携带者在平均年龄33岁时视力优于20/20，而受影响男性在30岁时这一比例为25%。
在患有CHM的受影响男性中，视力下降非常缓慢，直到患者达到50岁，此时视力丧失的速度和程度显著升高。此外，随着个体年龄增长，视力下降更快。与受影响男性不同，女性携带者的视力丧失要轻得多。
